URTICARIAL VASCULITIS.

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Hi anyone

Has anybody got any info or experience of Urticarial vasculitis pleas?

Is it linked to thyroid disorders ?

Thanks

Jean

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15 Replies

  • Posted

    Hi

    Urticarial vasculitis (also known as "Chronic urticaria as a manifestation of venulitis,

    "Hypocomplementemic urticarial vasculitis syndrome,

    "Hypocomplementemic vasculitis, and "Unusual lupus-like syndrome is a skin condition characterized by fixed urticarial lesions that appear histologically as a vasculitis.

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    • Posted

      Hi Jay

      I think that I had this in 2008 because I had three blisters which suddenly appeared on my left hand and abdomen leaving scars.

      I am now wondering if this was the real start of the thyroid disorder.

      Most of the other signs and symptoms started at this time but went worse in December 2013 and then my left leg swelled in March 2015. 

      ANAs done in August were 1 in 80 positive suggestive of SLE. However the Lupus anticoagulant test was negative. I still think that all of these issues point to an auto immune disease or attack on my thyroid. Does all this sound at all feasible?

      I am a novice to this mess.

      Jean x

       

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    • Posted

      Blisters ?

      Oh not shingles was it

      I got that right now 3rd time since xmas.

      They leave dark marks on skin.

      Shingles is a deep burning , burrowing pain, skin hurts to touch too.

      Jay x

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    • Posted

      jay

      THis is amazing, I was searching on the net to try to find a photo of the blister which came up on the back of my lefthand within minutes when I saw a photo of a cjicken pox lesion and also shingles. It dawned on me that I may have had chicken pox or shingles yet two senior drs did not even recognise the possible conditions. I then saw your e mail regarding the shingles!!!

      All this cannot just be sheer coincidence.By that I mean the apparent link/connection between skin' blisters' and the onset of these mismatch of signs and symptoms. These blisters were extremely painful and lasted a long time considering that they were mere blisters that one would get from say a scald.The scars remain especially on the back of my left hand. I had one blister on the back of my left hannd and two on my abdomen. Can this lead to peripheral neuropathy? Surely a dr could have diagnosed shingles etc?

      Jean

       

       

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    • Posted

      Jay

      Had to go to the desktop as new laptop playing up again.

      This is amazing, I was just about to reply when I received your last e mail re shingles, after looking up a picture of the blisters thast I had it dawned on me that I may have had either c/pox or shingles.

      I had two blisters on my abdomen and one sudden;y appear on the back of my left hand. I went to my former GPwho did not make any diagnosis just told the nurse to put a dressing on my hand. SHe gave me a supply of dressings to dress it myself.

      Cou;d al this have led to  /caused thyroid disorder plus the later development of peripheral neuropathy?

      I still have the scars and they are dark brown .The skin in tender but at the time and for quite a while afterwatrds was very sorem burning etc.

      I also had a 'blister' on my chest but that appeared a few months afterwards. The Practice Nurse said it was ringworm and prescribed an anti fungal which I am not allowed anyway. This took ages to clear up and in fact there is also a scar there but not as noticeable.

      Jean

      Jean

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    • Posted

      Hello Jean

      with my shingles , they are always in same place ..

      and before the blisters come out, theres a deep raw burning pain lasts for days and days, the skin is painful to even touch.. 

      If you take aciclovir tablets within 72 hours after the blisters ( like i did last time, chances are it will help and calm the virus .. 

      There may be long lasting pain after wards lasting weeks/months even years 

      Not for everyone .. 

      jay x

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    • Posted

      I do not know if shingles and thyroid are linked ..

      shingles ... 

      A blistering rash usually accompanies the outbreak.

      New blisters continue to appear for several days, and the entire outbreak can take weeks to heal.

      Blisters may cover the entire path of the affected nerve, or the involvement may be patchy.

      Involvement of the eyes during a shingles outbreak can be particularly serious, since scarring of the cornea and permanent vision loss can result.

      Another debilitating complication of shingles is known as postherpetic neuralgia.

      In postherpetic neuralgia, the pain of shingles persists even after the rash has healed.

      The diagnosis is made when pain lasts for over 30 days after an outbreak. This pain can last for weeks, months, or even years.

      Up to 15% of people with shingles have this persistent pain, and it is not understood why the pain persists in some people, but it is likely that some type of nerve damage or inflammation occurs during the outbreak that leads to continued pain.

      jay  

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    • Posted

      Jay

      The description etc certainly fits that of shingles. I have a pain along the right shoulder and sometimes along the nerve pathway of the left shouldrer blade as well.

      I have been to physio about this.

      s shingles then I feel very annoyed that the two drs I saw failed to recognise this.

      The photos certainly lead weight to the fact that this blistering was due to shingles.

      Could this have led to peripheral neuropathy and multinodular goitre?

      JeanIf what I had waa

       

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  • Posted

    Info of web

    Urticarial vasculitis is a clinico-pathologic entity typified by recurrent episodes of urticaria that have the histopathologic features of leukocytoclastic vasculitis. The cutaneous features may include painful, burning or pruritic skin lesions, the persistence of individual lesions greater than 24 hours, palpable purpura, pronounced central clearing of lesions, and residual hyperpigmentation following resolution.

    However, because clinical characteristics of urticarial vasculitis may overlap with those of allergic urticaria, confirmation of the diagnosis requires a lesional skin biopsy.

    This condition is idiopathic in many patients but can also occur in the context of autoimmune disorders, infections, drug reactions, or as a paraneoplastic syndrome.

    In idiopathic urticarial vasculitis common laboratory findings are an elevation of erythrocyte sedimentation rate and reduction of serum complement.

    An association between urticarial vasculitis and systemic lupus erythematosus has been hypothesized as some clinical manifestations of disease overlap and C1q autoantibodies may be present in both diseases.

    Normo-complementemic patients usually have minimal or no systemic involvement and often have a better prognosis.

    On-the-other-hand, hypocomplementemic patients have the propensity to have more severe multi-organ involvement.

    Response to treatment is variable and a wide variety of therapeutic agents may be efficacious.

    Initial recommendations for treatment of urticarial vasculitis manifest only as non-necrotizing skin lesions include antihistamines, dapsone, colchicine, hydroxychloroquine or indomethacin, but corticosteroids are often required.

    With necrotizing skin lesions or visceral involvement, corticosteroids are regularly indicated. Cases of severe corticosteroid resistant urticarial vasculitis or where corticosteroid morbidity is evident [table: see text] may require treatment with other immunosuppressive agents such as azathioprine, cyclophosphamide, or cyclosporine.

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  • Posted

    Info of web

    Urticarial vasculitis is a clinico-pathologic entity typified by recurrent episodes of urticaria that have the histopathologic features of leukocytoclastic vasculitis. The cutaneous features may include painful, burning or pruritic skin lesions, the persistence of individual lesions greater than 24 hours, palpable purpura, pronounced central clearing of lesions, and residual hyperpigmentation following resolution.

    However, because clinical characteristics of urticarial vasculitis may overlap with those of allergic urticaria, confirmation of the diagnosis requires a lesional skin biopsy.

    This condition is idiopathic in many patients but can also occur in the context of autoimmune disorders, infections, drug reactions, or as a paraneoplastic syndrome.

    In idiopathic urticarial vasculitis common laboratory findings are an elevation of erythrocyte sedimentation rate and reduction of serum complement.

    An association between urticarial vasculitis and systemic lupus erythematosus has been hypothesized as some clinical manifestations of disease overlap and C1q autoantibodies may be present in both diseases.

    Normo-complementemic patients usually have minimal or no systemic involvement and often have a better prognosis.

    On-the-other-hand, hypocomplementemic patients have the propensity to have more severe multi-organ involvement.

    Response to treatment is variable and a wide variety of therapeutic agents may be efficacious.

    Initial recommendations for treatment of urticarial vasculitis manifest only as non-necrotizing skin lesions include antihistamines, dapsone, colchicine, hydroxychloroquine or indomethacin, but corticosteroids are often required.

    With necrotizing skin lesions or visceral involvement, corticosteroids are regularly indicated. Cases of severe corticosteroid resistant urticarial vasculitis or where corticosteroid morbidity is evident [table: see text] may require treatment with other immunosuppressive agents such as azathioprine, cyclophosphamide, or cyclosporine.

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    • Posted

      Thanks Jay

      What am I going to do about this possible diagnosis .The blisters suddenly appeared for no reason withinh minutes. I showed them to the then GP and my neurologist. I was just told to attend the surgery for dressings.

      No tests were done for any inflammatory markers etc.

      All this happened in 2007/8.

      I will try to find a photo of these lesions.

      Jean

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  • Posted

    Urticarial Vasculitis

    What is Urticarial Vasculitis?

    Urticarial Vasculitis is a form of cutaneous vasculitis characterised by inflammation of the small blood vessels. Urticarial Vasculitis can be classified into three subtypes. All are defined by a measure of the "complement" levels in the blood. The complement system is a set of proteins that contribute to and amplify immune responses. They play a role in some, but not all, autoimmune disorders including some forms of Urticarial Vasculitis.

    Normocomplementemic Urticarial Vasculitis (NUV)

    NUV is diagnosed where a patient has the main symptoms of Urticarial Vasculitis combined with normal levels of C1q complements. NUV is generally the least severe form of Urticarial Vasculitis. It is less likely to be associated with any other symptoms.

    Hypocomplementemic Urticarial Vasculitis (HUV)

    HUV is diagnosed where the patient has the main symptoms of Urticarial Vasculitis combined with lower than normal levels of C1q complements and raised levels of anti-C1q antibodies (antibodies that attack the C1q complements). HUV is a more severe form of Urticarial Vasculitis and is likely to include symptoms such as purple or dark red spots or rash on the skin (a typical vasculitic rash); arthritic joint pain; breathing difficulties such as asthma, and stomach pains. Some regard HUV as a form of SLE (Lupus), although HUV patients usually test Anti-Nuclear Antibody (ANA) negative instead of the normal positive result for Lupus patients.

    Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)

    HUVS is a very rare and severe form of Urticarial Vasculitis. HUVS patients have more extensive complement abnormalities (low circulating 3rd and 4th complement components).

    As well as the symptoms of HUV, patients will suffer from systemic problems such as: episcleritis or uveitis (bloodshot or inflamed eyes) (found in 30 per cent of patients); mild glomerulonephritis (kidney disease); pleuritis (inflammation of the membrane surrounding the lungs); angioedema (swelling of the tissues under the skin) (found in 50 per cent of patients); Chronic Obstructive Pulmonary Disease (COPD) (breathing difficulties) (found in 50 per cent of patients), and cardiac involvement such as myocardial infarction (heart attack)

    Who are affected?

    NUV is most common between the ages of 30-40 and is found in women more than men. HUV and HUVS are almost exclusively found in women.

    What is the aetiology (cause)?

    The cause of Urticarial Vasculitis is not known in 50 per cent of cases. However it can be associated with or triggered by autoimmune/connective tissue diseases like Systemic Lupus Erythematosus (SLE or simply Lupus) Rheumatoid Arthritis, or occur in the context of a systemic vasculitis syndrome, such as Churg Strauss syndrome (Eosinophillic Granulomatosis with Polyangiitis); infections or viruses such as hepatitis; a reaction to certain drugs such as ACE inhibitors; certain types of diuretics; penicillin and Non-Steroidal Anti-Inflammatory Drugs (NSAIDs); cancer; and Glandular problems, such as Graves’ Disease (overactive thyroid).

    What are the symptoms?

    The main symptom is a recurring urticarial ("stinging nettle") rash that lasts for longer than 24 hours and "burns" rather than "itches", leaving marks behind on the skin when it clears.

    Diagnosis

    As in other types of vasculitis, diagnosis will depend on the doctor recognising the pattern of symptoms and examination findings. Blood is commonly taken to test for raised levels of "C-reactive protein" (CRP) and "Erythrocyte Sedimentation Rate" (ESR) which indicate inflammation in the body. Skin and kidney biopsies may also be taken to confirm the diagnosis.

    Testing for C1q, anti-C1q, C3 and C4 complement components is essential to determine the type of Urticarial Vasculitis.

    Tests may also be done for Anti-Nuclear Antibody (ANA) levels (which are positive in 30 to 50 per cent of patients), and Anti ds-DNA levels may also be positive.

    Treatment

    Milder cases of Urticarial Vasculitis may simply be treated with antihistamines and NSAIDs such as Ibuprofen. Corticosteroids such as Prednisolone might be used for more persistent cases. However for the more severe forms of Urticarial Vasculitis, steroids such as Prednisolone are usually prescribed to reduce inflammation as well as immunosuppressants such as Azathioprine, Cyclophosphamide or Mycophenolate Mofetil (CellCept).

    In cases where a patient is unresponsive to treatment, intravenous immunoglobulin and anti-cytokine monoclonal antibodies or rituximab may have a role.

    If the disease is very severe large doses of Methylprednisolone or Plasmapheresis (plasma exchange) may also be given. When the disease becomes quiet less toxic drugs are used to keep control and these include: Azathioprine, Methotrexate and Mycophenolate Mofetil usually in combination with low dose prednisolone.

    Drugs and Side Effects

    For information on the main drugs prescribed for Urticarial Vasculitis see:

    Azathioprine

    Cyclophosphamide

    Methotrexate

    Mycophenolate Mofetil (Cellcept)

    Rituximab

    Steroids

    For information on other drugs used in the treatment of vasculitis see Glossary of drugs and side effects.

    Treatments - Procedures

    Plasma exchange or plasmapheresis

    This treatment is sometimes used in patients with severe vasculitis where antibodies in the blood are thought to be important in causing the disease. The treatment involves removing antibodies from the blood using a machine and returning the "cleaned" blood back to the patient. The treatment may necessitate giving blood products to the patient including plasma, albumin or immunoglobulin. It may also involve giving drugs to thin the blood and prevent it clotting in the machine.

    For information on plasma exchange:

    Plasma exchange or plasmapheresis

    Prognosis

    The overall prognosis in Urticarial Vasculitis depends on the severity of the disease and the amount of damage that has been done to organs, especially the lungs. The main risk to patients appears to be Chronic Obstructive Pulmonary Disease (COPD).

    Smoking is a major risk factor for fatal lung disease in HUVS and smokers should seek help to give up as soon as possible.

    Key Points

    The systemic HUVS type of Urticarial Vasculitis is a very rare form of the disease

    The main symptom is a recurring urticarial ("stinging nettle") rash

    The levels of C1q complements in the blood are a significant indicator for diagnosis and treatment

    Treatment depends upon the severity of the disease

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  • Posted

    hi Jean. Urticarial vasculitis is sometimes a symptom of wider vasculitis and yes vasculitis can attack the thyroid. Either over or underactive thyroid can be attributed to vasculitis. I am not sure if you are still logging into the site but if you want to chat about it please do. I have vasculitis and my thyroid has been borderline sometimes but never low enough to cause concern. 

    Obviously vasculitis is not always the cause of thyroid problems but it certainly can affect the thyriod gland. 

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